Chapter XVII
Congenital malformations, deformations and chromosomal abnormalities
(Q00-Q99)

Congenital malformations of genital organs
(Q50-Q56)

Excl.:
androgen resistance syndrome ( E34.5)
syndromes associated with anomalies in the number and form of chromosomes ( Q90-Q99)
testicular feminization syndrome ( E34.5)

Q50 Congenital malformations of ovaries, fallopian tubes and broad ligaments

Q50.0 Congenital absence of ovary
Excl.:
Turner syndrome ( Q96.-)
Q50.1 Developmental ovarian cyst
Q50.2 Congenital torsion of ovary
Q50.3 Other congenital malformations of ovary
Incl.:
Accessory ovary
Congenital malformation of ovary NOS
Ovarian streak
Q50.4 Embryonic cyst of fallopian tube
Incl.:
Fimbrial cyst
Q50.5 Embryonic cyst of broad ligament
Incl.:
Cyst:
  • epoophoron
  • Gartner duct
  • parovarian
Q50.6 Other congenital malformations of fallopian tube and broad ligament
Incl.:
  • Absence
  • Accessory
  • Atresia
  • (of) fallopian tube or broad ligament
Congenital malformation of fallopian tube or broad ligament NOS

Q51 Congenital malformations of uterus and cervix

Q51.0 Agenesis and aplasia of uterus
Incl.:
Congenital absence of uterus
Q51.1 Doubling of uterus with doubling of cervix and vagina
Q51.2 Other doubling of uterus
Incl.:
Doubling of uterus NOS
Q51.3 Bicornate uterus
Q51.4 Unicornate uterus
Q51.5 Agenesis and aplasia of cervix
Incl.:
Congenital absence of cervix
Q51.6 Embryonic cyst of cervix
Q51.7 Congenital fistulae between uterus and digestive and urinary tracts
Q51.8 Other congenital malformations of uterus and cervix
Incl.:
Hypoplasia of uterus and cervix
Q51.9 Congenital malformation of uterus and cervix, unspecified

Q52 Other congenital malformations of female genitalia

Q52.0 Congenital absence of vagina
Q52.1 Doubling of vagina
Incl.:
Septate vagina
Excl.:
doubling of vagina with doubling of uterus and cervix ( Q51.1)
Q52.2 Congenital rectovaginal fistula
Excl.:
cloaca ( Q43.7)
Q52.3 Imperforate hymen
Q52.4 Other congenital malformations of vagina
Incl.:
Congenital malformation of vagina NOS
Cyst:
  • canal of Nuck, congenital
  • embryonic vaginal
Q52.5 Fusion of labia
Q52.6 Congenital malformation of clitoris
Q52.7 Other congenital malformations of vulva
Incl.:
  • Congenital:
    • absence
    • cyst
    • malformation NOS
  • of vulva
Q52.8 Other specified congenital malformations of female genitalia
Q52.9 Congenital malformation of female genitalia, unspecified

Q53 Undescended testicle

Q53.0 Ectopic testis
Incl.:
Unilateral or bilateral ectopic testes
Q53.1 Undescended testicle, unilateral
Q53.2 Undescended testicle, bilateral
Q53.9 Undescended testicle, unspecified
Incl.:
Cryptorchism NOS

Q54 Hypospadias

Excl.:
epispadias ( Q64.0)
Q54.0 Hypospadias, balanic
Incl.:
Hypospadias:
  • coronal
  • glandular
Q54.1 Hypospadias, penile
Q54.2 Hypospadias, penoscrotal
Q54.3 Hypospadias, perineal
Q54.4 Congenital chordee
Q54.8 Other hypospadias
Q54.9 Hypospadias, unspecified

Q55 Other congenital malformations of male genital organs

Excl.:
congenital hydrocele ( P83.5)
hypospadias ( Q54.-)
Q55.0 Absence and aplasia of testis
Incl.:
Monorchism
Q55.1 Hypoplasia of testis and scrotum
Incl.:
Fusion of testes
Q55.2 Other congenital malformations of testis and scrotum
Incl.:
Congenital malformation of testis or scrotum NOS
Polyorchism
Retractile testis
Testis migrans
Q55.3 Atresia of vas deferens
Q55.4 Other congenital malformations of vas deferens, epididymis, seminal vesicles and prostate
Incl.:
Absence or aplasia of:
  • prostate
  • spermatic cord
Congenital malformation of vas deferens, epididymis, seminal vesicles or prostate NOS
Q55.5 Congenital absence and aplasia of penis
Q55.6 Other congenital malformations of penis
Incl.:
Congenital malformation of penis NOS
Curvature of penis (lateral)
Hypoplasia of penis
Q55.8 Other specified congenital malformations of male genital organs
Q55.9 Congenital malformation of male genital organ, unspecified
Incl.:
  • Congenital:
    • anomaly
    • deformity
  • NOS of male genital organ

Q56 Indeterminate sex and pseudohermaphroditism

Excl.:
pseudohermaphroditism:
  • female, with adrenocortical disorder (E25.-)
  • male, with androgen resistance (E34.5)
  • with specified chromosomal anomaly (Q96-Q99)
Q56.0 Hermaphroditism, not elsewhere classified
Incl.:
Ovotestis
Q56.1 Male pseudohermaphroditism, not elsewhere classified
Incl.:
Male pseudohermaphroditism NOS
Q56.2 Female pseudohermaphroditism, not elsewhere classified
Incl.:
Female pseudohermaphroditism NOS
Q56.3 Pseudohermaphroditism, unspecified
Q56.4 Indeterminate sex, unspecified
Incl.:
Ambiguous genitalia